Rare immunodeficiency provides new insight into immune system function

Scientists now have a better understanding of the complex events that contribute to the pathology of an inherited immunodeficiency called X-linked lymphoproliferative disease (XLP). Two separate research studies investigating this rare disorder provide critical new information about the regulation of T helper cell cytokine secretion, a process that is absolutely critical for appropriate immune responses to infectious agents. The research is published in the November issue of Immunity.

XLP is a rare, inherited immunodeficiency that is characterized by an abnormal and extreme sensitivity to the Epstein-Barr virus (EBV). XLP patients exposed to EBV have a severe and often fatal massive proliferation of abnormal T lymphocytes. Those individuals who survive the EBV infection have a significant risk of developing lymphomas. XLP is caused by mutation of a gene expressed in T cells that encodes a protein called SAP. Although the precise dysregulation of T cells that underlies XLP is unclear, research using genetically engineered mice that lack SAP demonstrated that T helper cells in these animals exhibit reduced cytokine production.

Dr. Pamela L. Schwartzberg from the National Human Genome Research Institute and colleagues used SAP-deficient T cells to investigate specific aspects of the T cell signaling cascade. Some components of the signaling cascade used by activated T cells are unperturbed in the mutant T cells whereas other aspects are impaired. Dr. Schwartzberg's group dissects specific T cell signaling molecules, such as Fyn, that are linked to reduced cytokine production in the SAP mutants. "Our data argue that a SAP/Fyn pathway may be required for the efficient recruitment of PKC-q and Bcl-10, as well as proper patterns of activation of NF-kB, and suggest a potentially novel pathway of T helper cell cytokine activation," offers Dr. Schwartzberg.

A separate study led by Dr. Andre Veillette from the Institut de recherches cliniques de Montrea

Contact: Heidi Hardman
Cell Press

Page: 1 2

Related biology news :

1. Heavy drinking can hasten the progression of the simian immunodeficiency virus disease
2. Encouraging results of gene therapy for severe combined immunodeficiency
3. Female gender provides an advantage in renal diseases
4. Forecasting system provides flood warnings to vulnerable residents of Bangladesh
5. Genomics study provides insight into the evolution of unique human traits
6. Study provides new data about the laws governing embryo development in organisms
7. Sea anemone genome provides new view of our multi-celled ancestors
8. Latest data shows MabThera provides significant, sustained relief from symptoms of RA
9. Understanding the global carbon budget -- Woods Hole Research Center expert provides insights
10. Nanotechnology provides green path to environmentally sustainable economy
11. American Association for Cancer Research provides support for promising cancer scientists

Post Your Comments:

(Date:7/18/2020)... ... July 16, 2020 , ... ... effectiveness of the Invictus Medical Neoasis™ active noise control device to attenuate typical ... that the Neoasis™ device attenuated the alarm sounds from patient monitors, ventilators and ...
(Date:7/4/2020)... ... 2020 , ... Aesthetics Biomedical (ABM), a leading aesthetics product ... only the products and treatments developed, but also the dedicated team behind the ... brands built by ABM have received several honors already for 2020, each of ...
(Date:7/1/2020)... VENICE, Calif. (PRWEB) , ... July 01, 2020 ... ... focused on building awareness and solutions for glioblastoma—the most common and aggressive adult ... and Lisa Haile, JD, PhD. Senior Fellows are charged with supporting the organization’s ...
Breaking Biology News(10 mins):
(Date:7/31/2020)... ... ... eSource has long been touted as the solution to high data management ... reasons it did not take off as quickly as people initially expected, and where ... towards capturing data electronically for clinical trials and then repurposing it for downstream analysis, ...
(Date:7/31/2020)... ... July 30, 2020 , ... ... today announced the release of its signature product called Beacon - a ... and other field medical professionals. , Beacon helps MSLs streamline communication ...
(Date:7/18/2020)... ... ... After research model surgery and catheter implantation, the long journey of catheter ... of a successful study, while protecting and accessing the catheter or device post-surgery is ... from Envigo in a live webinar on Wednesday, August 5, ...
(Date:7/18/2020)... ... July 16, 2020 , ... Medial ... early detection and prevention of high-burden diseases, and Centric Consulting, a business and ... organizations to utilize existing data in order to identify and prioritize patients for ...
Breaking Biology Technology:
Cached News: